3-Methoxytyrosine

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3-Methoxytyrosine
2D structure for 3-Methoxytyrosine
Chemical Name	2-amino-3-(4-hydroxy-3-methoxy-phenyl)propanoic acid
Chemical Formula	C₁₀H₁₃NO₄
CAS Number	7636-26-2
Chemical Information	HMDB01434
Biochemical Taxonomy	Amino Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood Cerebrospinal Fluid (CSF)
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 0.089 +/- 0.032 umol/L Cerebrospinal Fluid (CSF): 0.025 +/-0.025 (0 - 0.05) uM
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Aromatic L-Amino Acid Decarboxylase (AADC) Deficiency Inborn error of aromatic L-amino acid decarboxylase
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Cerebrospinal Fluid (CSF) (Aromatic L-Amino Acid Decarboxylase (AADC) Deficiency): 1.2 (1.0 - 1.6) uM Cerebrospinal Fluid (CSF) (Inborn error of aromatic L-amino acid decarboxylase): uM
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
3-Methoxytyrosine is one of the main biochemical markers for Aromatic L-amino acid decarboxylase (AADC , EC 4.1.1.28) deficiency, an inborn error of metabolism that affects serotonin and dopamine biosynthesis. Patients are usually detected in infancy due to developmental delay, hypotonia, and extrapyramidal movements. Diagnosis is based on an abnormal neurotransmitter metabolite profile in CSF and reduced AADC activity in plasma. 3-methoxytyrosine is elevated in CSF, plasma, and urine. (PMID 1357595, 1281049, 16288991)